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Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age.
[pleomorphic liposarcoma]
Liposarcomas
typically
occur
in
middle
aged
to
older
adults
.
Altogether
,
approximately
50
bona
fide
liposarcomas
have
been
reported
in
children
and
adolescents
,
most
of
which
have
represented
myxoid
liposarcomas
,
with
a
good
prognosis
.
We
undertook
a
retrospective
study
of
82
liposarcomas
occurring
in
patients
below
22
years
of
age
.
Clinicopathologic
and
follow-up
information
was
obtained
.
Fluorescence
in
situ
hybridization
for
FUS
,
EWSR
1
,
CHOP
(
DDIT
3
)
,
and
MDM
2
was
performed
in
30
cases
.
The
tumors
occurred
in
28
males
and
54
females
(
5
to
22
y
of
age
)
and
involved
many
locations
.
Fifty
-
six
cases
were
typical
myxoid
liposarcomas
,
including
2
with
round
cell
areas
.
The
tumors
were
grade
1
(
56
cases
)
and
grade
3
(
2
cases
)
.
Thirty
-
seven
of
38
patients
with
follow-up
are
alive
without
disease
and
1
is
alive
with
disease
(
median
59
mo
follow-up
duration
,
range
:
8
to
108
mo
)
.
Six
cases
showed
myxoid
liposarcoma
with
spindled
growth
(
"
spindle
cell
myxoid
liposarcoma
"
)
;
these
arose
in
5
females
and
1
male
(
median
age
14
y
)
and
involved
the
thigh
in
40
%
of
cases
.
All
were
grade
1
.
Follow-up
(
4
of
6
patients
)
showed
local
recurrences
in
2
cases
and
metastases
in
1
case
.
Twelve
tumors
consisted
of
conventional
myxoid
liposarcoma
and
pleomorphic
liposarcoma
(
"
pleomorphic
myxoid
liposarcoma
"
)
;
these
arose
in
4
males
and
8
females
(
10
to
22
y
of
age
)
and
often
involved
the
mediastinum
.
Tumor
grades
were
2
(
4
cases
)
and
3
(
8
cases
)
.
Follow-up
(
10
patients
)
showed
7
dead
of
disease
,
1
alive
with
disease
,
and
2
disease
free
.
Four
atypical
lipomatous
tumors
were
seen
including
2
with
low
-grade
dedifferentiation
.
Two
local
recurrences
were
seen
;
all
patients
are
disease
free
.
Two
conventional
pleomorphic
liposarcomas
were
seen
;
1
patient
with
follow-up
is
disease
free
.
FUS
-CHOP
and
EWSR
1
-
CHOP
rearrangements
were
identified
by
fluorescence
in
situ
hybridization
in
15
/
23
and
2
/
23
conventional
myxoid
liposarcomas
,
respectively
,
and
in
no
other
tumors
.
Amplification
for
MDM
2
was
absent
in
all
cases
.
We
conclude
that
conventional
myxoid
liposarcoma
is
by
far
the
most
common
subtype
of
liposarcoma
in
young
patients
,
with
an
excellent
prognosis
.
Two
apparently
novel
subtypes
of
liposarcoma
,
termed
pleomorphic
myxoid
liposarcoma
and
spindle
cell
myxoid
liposarcoma
comprise
considerable
percentages
of
liposarcomas
in
this
age
group
and
should
be
distinguished
from
conventional
myxoid
liposarcoma
and
conventional
pleomorphic
liposarcoma
.
Pleomorphic
myxoid
liposarcoma
and
spindle
cell
myxoid
liposarcoma
most
likely
represent
high
-grade
and
low
-grade
variants
of
myxoid
liposarcoma
,
respectively
.
Additional
study
of
such
cases
will
be
necessary
for
definitive
classification
.
Diseases
Validation
Diseases presenting
"low-grade dedifferentiation"
symptom
pleomorphic liposarcoma
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